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ORIGINAL ARTICLE
Year : 2015  |  Volume : 56  |  Issue : 3  |  Page : 204-207  

Adolescents with sickle cell anaemia: Experience in a private tertiary hospital serving a tertiary institution


Department of Haematology, Ben Carson School of Medicine, Babcock University, Ogun, Nigeria

Date of Web Publication9-Jul-2015

Correspondence Address:
Sarah John-Olabode
Department of Haematology, Ben Carson School of Medicine, Babcock University, Ogun
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0300-1652.160397

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   Abstract 

Background: Many adolescents with sickle cell disease (SCD) have adjustment difficulties in the transition period from paediatric care to the adult system because they find themselves in unfamiliar waters where they have to learn to manage themselves. The aim of this study is to evaluate the prevalent crises and morbidities associated with SCD in adolescents in Babcock University Teaching Hospital (BUTH), to also assess the level of knowledge of these adolescents about SCD and to determine their emotional response to the disease. Materials and Methods: This was a retrospective review of case notes of adolescents with sickle cell anaemia that were seen in BUTH, from May 2013 to April 2014. Data extracted from the case notes was entered into a Microsoft (MS) Excel and analysed using descriptive statistics. Results were presented in tables. Results: A total of 50 subjects were seen in the department during this study period. Vaso-occlusive crises in the form of bone pains (93.1%) were the commonest crises encountered. Associated morbidities were malaria 34 (85%), tonsilitis 1 (2.5%), pneumonia 1 (2.5%), leg ulcer 1 (2.5%), azotaemia 1 (2.5%) and subarachnoid haemorrhage 2 (5%). Majority (88%) had adequate knowledge about general health maintenance while knowledge on nutrition and appropriate analgesia use is still inadequate. Eleven (22%) had symptoms of depression, four (8%) had suicidal ideation while one (2%) had a history of attempted suicide. Conclusion: This study emphasizes the importance of psychosocial intervention as part of a comprehensive health management for people with SCD.

Keywords: Adolescent, psychosocial intervention, sickle cell anaemia


How to cite this article:
John-Olabode S, Awodele I, Oni O. Adolescents with sickle cell anaemia: Experience in a private tertiary hospital serving a tertiary institution. Niger Med J 2015;56:204-7

How to cite this URL:
John-Olabode S, Awodele I, Oni O. Adolescents with sickle cell anaemia: Experience in a private tertiary hospital serving a tertiary institution. Niger Med J [serial online] 2015 [cited 2019 Dec 13];56:204-7. Available from: http://www.nigeriamedj.com/text.asp?2015/56/3/204/160397


   Introduction Top


The commonest genetic disorder among Africans is sickle cell disease (SCD). [1],[2] In Nigeria, the prevalence of sickle cell trait is about 25% while the homozygous state is found in about 3% of the population. [3] Nigeria has the largest population of people with SCD, with about 150,000 births annually. [4],[5]

The aim of this study is to evaluate the prevalent crises and morbidities associated with SCD in adolescents in Babcock University Teaching Hospital (BUTH), to also assess the level of knowledge of these adolescents about SCD and to determine their emotional response to the disease.


   Materials and methods Top


This was a retrospective review of case notes of adolescents with sickle cell anaemia (aged 17-24 years) that was seen in the emergency room and Haematology Clinic of BUTH, Ogun State, Nigeria from May 2013 to April 2014. The Haematology Clinic of BUTH operates once a week and receives referrals from the Babcock University, other units within the Clinical Department in the hospital and its environs. Only adolescents with haemoglobin genotype SS established by the cellulose acetate haemoglobin electrophoresis were included for this study. Those with incomplete data were excluded. Data extracted from the patients' case notes included psychological assessment, age, gender, genotype, history of blood transfusion, complications and crises.

Data was entered into a Microsoft (MS) Excel Spread sheet and analysed using descriptive statistics. Results were presented in tables.


   Result Top


A total of 50 adolescents were seen in the hospital during this study period. Of these 50 (aged 17-24 years), 25 (50%) were males while 25 (50%) were females giving a male/female ratio of 1:1. The mean age was 21.2 ± 5.2 years. Forty-nine (98%) were students while one (2%) subject a nurse [Table 1].
Table 1: Socio-demographic data

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Vaso-occlusive crises (VOC) in the form of bone pains (93.1%) were the commonest crises encountered [Table 2]. In total, 108 episodes of bone pain were documented giving an average rate of 2.6 episodes per patient. The average duration for each episode was about 7 days.
Table 2: Type of cickle cell crises

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Three (6%) adolescents gave a history of previous blood transfusions (ranging from one to two). Associated morbidities were malaria 34 (85%), tonsilitis 1 (2.5%), pneumonia 1 (2.5%), leg ulcer 1 (2.5%), azotaemia 1 (2.5%) and subarachnoid haemorrhage 2 (5%) [Figure 1].
Figure 1: Morbidities associated with SCA

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Majority (88%) had adequate knowledge about general health maintenance while knowledge on nutrition and appropriate analgesia use is still largely inadequate [Table 3]. Eleven (22%) had symptoms attributable to depression, four (8%) had suicidal ideation while one (2%) had a history of attempted suicide.
Table 3: Subjects' knowledge about sickle cell anaemia

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   Discussion Top


Previous reports have described SCD as a condition of childhood with most youths not living past their teens. [6],[7] Improvement in the diagnosis and treatment of many of SCD's life-threatening conditions over the past decade has led to an extended life expectancy with many living well into their 40s. [8] Many adolescents with SCD have to deal the psychological burden of having a chronic disease, the disadvantage of being categorised as socially dysfunctional. [9],[10] As a result, there is clinical consensus that added attention needs to be focused on the period from adolescence to young adulthood.

To facilitate adolescents with SCD live a longer and healthier life, they should be taught to manage their illness. LePontois and others [11],[12],[13],[14] are of the opinion that there is need for adolescents with chronic conditions, like SCD, to develop both short- and long-term psychological and social skills needed to successfully navigate this developmental period and the medical and social systems in which they function.

Acute episodes of bone pain signalling marrow ischaemia or necrosis is pathognomonic of SCD and is the most common cause of hospital admissions. These recurrent attacks of acute pain are serious complications that often require treatment with parenteral opioids, in the Emergency Room (ER) and/or the hospital. [15],[16],[17]

This study reveals that bone pain is the commonest crises necessitating hospital admission as documented in previous studies. [18],[19] The episode of pain per patient is higher than that documented by Platt et al.; this could be a consequence of the academic stress the subjects are experiencing. However, the average duration of pain of 7 days is consistent with literature. [15]

In this study, the commonest indication for blood transfusion was severe anaemia; this is consistent with the finding of Otaigbe in Port Harcourt, Nigeria. [20] This is however different from studies in developed countries where the indication for transfusion were variable with several studies showing a decline in complication rates among sickle cell patients. [21],[22]

Nigeria is a malaria-endemic region; so, it is not surprising that the prevalent morbidity recorded in these subjects was malaria. This finding is also consistent with results from other studies. [18],[23] The fact that malaria infection precipitates VOC could also account for bone pain crises being the commonest presentation of sickle cell crises recorded in this study.

It was quite interesting to note that majority of the subjects had adequate knowledge about general health maintenance; though on further questioning, many of the subjects do not actually stick to their care plan. The reluctance to use routine medications, to avoid conditions that can precipitate VOC and attend follow-up appointments is all part of the adjustment difficulties documented in chronic medical conditions like SCD. [24],[25],[26]

Consistent with the literature, [4],[27],[28],[29] a significant number of the patients in this study had feelings of depression. However, it was noted that subjects who had more knowledge about their condition with strong family support had a more positive outlook about their disease; improved coping skills and better adherence to their routine medications which could be responsible for reduced hospital visits.


   Conclusion Top


The study shows that the majority of adolescents who have better knowledge about their condition with strong family support have a positive perception of their illness which equates not only better control of their illness but also better quality of life with reduced hospital visits. This study emphasizes the importance of psychosocial intervention as part of a comprehensive health management for people with SCD.

The onus is on health-care providers to work closely with the family to ensure that there is a smooth transition from paediatric to adolescent care by providing as much information as possible to assist the affected adolescent in navigating this turbulent period.

 
   References Top

1.
Akinyanju OO. A profile of sickle cell disease in Nigeria. Ann N Y Acad Sci 1989;565:126-36.  Back to cited text no. 1
    
2.
WHO. Proposal for a feasibility study on the control of sickle cell disease in Africa. Report of a WHO informal consultation, Geneva. 6-7 [th] February 1987. Geneva: WHO, 1987.  Back to cited text no. 2
    
3.
Adekile AD, Adeodu OO. Haemoglobinopathies. In: Azubuike JC, Nkanginieme KE, editors. Textbook of Paediatrics and Child Health in a Tropical Region. 2 [nd] ed. Owerri: African Educational Services; 2007. p. 373-90.  Back to cited text no. 3
    
4.
Anie KA, Egunjobi FE, Akinyanju OO. Psychosocial impact of sickle cell disorder: Perspectives from a Nigerian setting. Global Health 2010;6:2.  Back to cited text no. 4
    
5.
World Health Organization. Report by the Secretariat of the Fifty-ninth World Health Assembly A59/9; 2006.  Back to cited text no. 5
    
6.
Dacie JV. The Haemolytic Anaemias: Congenital and Acquired, Part I: The Congenital Anaemias. 2nd ed. New York: Grune & Stratton; 1960.  Back to cited text no. 6
    
7.
Diggs LM. Anatomic lesions in sickle cell disease. In: Abramson H, Bertles JF, Whethers DL, editors. Sickle Cell Disease Diagnosis, Management, Education and Research. St. Louis: C.V. Mosby Co; 1973. p. 189-229.  Back to cited text no. 7
    
8.
Scott RB. Advances in the treatment of sickle cell disease in children. Am J Dis Child 1985;139:1219-22.  Back to cited text no. 8
    
9.
Vavasseur JW. Psychological aspects of chronic disease: Cultural and ethnic implications. Birth Defects Orig Artic Ser 1987;23:144-53.  Back to cited text no. 9
    
10.
Telfair J, Nash KB. Delivery of genetic services to African Americans. In: Fisher NL, editor. Ethnic and Cultural Diversity and its Impact on the Delivery of Genetic Services. Baltimore: Johns Hopkins University Press, 1996; 35-59   Back to cited text no. 10
    
11.
LePontois J. Adolescents with sickle cell anaemia: Developmental issues. In: Hurtig AL, Viera CT, editors. Sickle Cell Disease: Psychological and Psychosocial Issues. Urbana and Chicago: University of Illinois Press; 1986. p. 75-83.  Back to cited text no. 11
    
12.
Gil KM, Wilson JJ, Edens JL. The stability of pain coping strategies in young children adolescents, and adults with sickle cell disease over an 18-month period. Clin J Pain 1997;13:110-5.  Back to cited text no. 12
    
13.
Thompson RJ Jr, Armstrong FD, Kronenberger WG, Scott D, McCabe MA, Smith B, et al. Family functioning, neurocognitive functioning, and behavior problems in children with sickle cell disease. J Pediatr Psychol 1999;4:491-8.  Back to cited text no. 13
    
14.
Weitzman M. School and peer relations. Pediatr Clin North Am 1984;31:59-69.  Back to cited text no. 14
    
15.
Ballas SK. Sickle cell pain. Progress in Pain Research and Management. Vol. 11. Seattle: IASP Press; 1998.  Back to cited text no. 15
    
16.
Ballas SK. Update on pain management in sickle cell disease. Hemoglobin 2011;35:520-9.  Back to cited text no. 16
    
17.
Mankard VN, Williams JP, Harpen MD, Manci E, Longenecker G, Moore RB, et al. Magnetic resonance imaging of bone marrow in sickle cell disease: Clinical, hematologic, and pathologic correlations. Blood 1990;75:274-83.  Back to cited text no. 17
    
18.
Innocent Ocheyana George, Peace Ibo Opara. Sickle Cell Anaemia: A survey of associated morbidities in Nigerian children. Afr J Haematol Oncol 2011;2:187-90.  Back to cited text no. 18
    
19.
Platt OS, Thorington BD, Brambilla DJ, Milner PF, Rosse WF, Vichinsky E, et al. Pain in sickle cell disease. N Engl J Med 1991;325:11-6.  Back to cited text no. 19
    
20.
Otaigbe B. Prevalence of blood transfusion in sickle cell anaemia patients in South-South Nigeria: A two-year experience. Int J Med Med Sci Res 2013;1:013-8.  Back to cited text no. 20
    
21.
Ware RE, Helms RW. SWiTCH Investigators. Stroke with transfusions changing to hydroxyurea (SWiTCH). Blood 2012;119:3925-32.  Back to cited text no. 21
    
22.
Cheung TW, Miller JW, Miguelino MG, To WJ, Li J, Lin X, et al. Exchange transfusion therapy and its effects on real-time microcirculation in pediatric sickle cell anemia patients: An intravital microscopy study. J Pediatr Hematol Oncol 2012;34:169-17.  Back to cited text no. 22
    
23.
Fleming AF. The presentation, management and prevention of crisis in sickle cell disease in Africa. Blood Rev 1989;3:18-28.  Back to cited text no. 23
    
24.
Barlow JH, Ellard DR. The psychosocial well-being of children with chronic disease, their parents and siblings: An overview of the research evidence base. Child Care Health Dev 2006;32:19-31.  Back to cited text no. 24
    
25.
Midence K, Fuggle P, Davies SC. Psychosocial aspects of sickle cell disease (SCD) in childhood and adolescence: A review. Br J Clin Psychol 1993;32:271-80.  Back to cited text no. 25
    
26.
Gortmaker SL, Walker DK, Weitzman M, Sobol AM. Chronic conditions, socioeconomic risks, and behavioral problems in children and adolescents. Paediatrics 1990;85:267-76.  Back to cited text no. 26
    
27.
Ohaeri JU, Shokunbi WA, Akindale KS, Dare LO. The psychosocial problems of sickle cell disease sufferers and their methods of coping. Soc Sci Med 1995;40:955-60.  Back to cited text no. 27
    
28.
Yang YM, Cepeda M, Price C, Shah A, Mankad V. Depression in children and adolescents with sickle-cell disease. Arch Pediatr Adolesc Med 1994;148:457-60.  Back to cited text no. 28
    
29.
Hasan SP, Hashmi S, Alhassen M, Lawson W, Castro O. Depression in sickle cell disease. J Natl Med Assoc 2003; 95:533-7.  Back to cited text no. 29
    


    Figures

  [Figure 1]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]


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[Pubmed] | [DOI]



 

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